Hepatocellular carcinoma(HCC)is rarely associated with autoimmune paraneoplastic syndromes.We report a case of anti-transcriptional intermediary factor-1 gamma(TIF1-γ)-positive dermatomyositis(DM)as clinical presenta...Hepatocellular carcinoma(HCC)is rarely associated with autoimmune paraneoplastic syndromes.We report a case of anti-transcriptional intermediary factor-1 gamma(TIF1-γ)-positive dermatomyositis(DM)as clinical presentation of HCC recurrence in a 72-year-old male patient admitted to our hospital due to fatigue,myalgia,and typical skin rash.His medical history was notable for hepatitis C-related cirrhosis,successful treatment with direct-acting antiviral agents,and previously efficacious treatment of HCC.Laboratory testing showed significant rhabdomyolysis with antiTIF1-γantibodies at high titer,and DM was diagnosed.After a careful diagnostic workup,HCC recurrence was diagnosed.After first-line corticosteroid treatment,azathioprine and intravenous immunoglobulin treatments were administered;unfortunately,he mounted only partial response.Owing to the compromised performance status,no HCC treatment was feasible,and,according to international guidelines,he received only best supportive care.Here,we discuss the diagnostic,prognostic,and pathogenic roles of anti-TIF1-γantibodies associated with paraneoplastic DM and the scant literature data on its occurrence in HCC patients.Considering the TIF1 gene family’s established role in oncogenesis,we also review the role of TIF1-γas a tumor-related neoantigen,leading to the development of clinically overt antiTIF1-γantibodies-positive DM.展开更多
文摘Hepatocellular carcinoma(HCC)is rarely associated with autoimmune paraneoplastic syndromes.We report a case of anti-transcriptional intermediary factor-1 gamma(TIF1-γ)-positive dermatomyositis(DM)as clinical presentation of HCC recurrence in a 72-year-old male patient admitted to our hospital due to fatigue,myalgia,and typical skin rash.His medical history was notable for hepatitis C-related cirrhosis,successful treatment with direct-acting antiviral agents,and previously efficacious treatment of HCC.Laboratory testing showed significant rhabdomyolysis with antiTIF1-γantibodies at high titer,and DM was diagnosed.After a careful diagnostic workup,HCC recurrence was diagnosed.After first-line corticosteroid treatment,azathioprine and intravenous immunoglobulin treatments were administered;unfortunately,he mounted only partial response.Owing to the compromised performance status,no HCC treatment was feasible,and,according to international guidelines,he received only best supportive care.Here,we discuss the diagnostic,prognostic,and pathogenic roles of anti-TIF1-γantibodies associated with paraneoplastic DM and the scant literature data on its occurrence in HCC patients.Considering the TIF1 gene family’s established role in oncogenesis,we also review the role of TIF1-γas a tumor-related neoantigen,leading to the development of clinically overt antiTIF1-γantibodies-positive DM.